Extramedullary Intradural Primary Spinal Angiosarcoma: A Case Study

Angiosarcoma is a rare soft tissue sarcoma, namely when it presents as a primary intradural extramedullary spinal neoplasm, with only one case of non-vertebral origin reported in the literature. We present the case of a 51-year-old woman with neurological symptoms of paraparesis and constipation who underwent a magnetic resonance imaging (MRI) that revealed a well-demarcated, predominantly homogeneous, intensely enhancing intradural extramedullary lesion in T2-weighted images. Histology, immunohistochemistry, and genetics of the lesion showed an angiosarcoma. The patient received adjuvant radiation therapy (RT) and is currently stable, showing no signs of recurrence, in the third year of follow-up. Our work demonstrates a rare case of extramedullary intradural primary spinal angiosarcoma, highlighting the challenges associated with its diagnosis. The multidisciplinary approach, including surgery and adjuvant RT, has been shown to effectively control the disease. Although the prognosis is generally poor, the presented case shows apparent success.